title: Transarterial treatment of congenital renal arteriovenous fistulas.
abstract: OBJECTIVE
  Congenital renal arteriovenous fistulas (CRAVF) represent a distinct clinical entity with characteristic hemodynamic and angiographic features. Treatment is warranted given potential for growth with renal and hemodynamic compromise. We report our experience in a rare series of treated symptomatic CRAVFs.
  
  
  METHODS
  Over a 10-year period, patients treated for symptomatic CRAVFs (no history of predisposing renal pathology, instrumentation, neoplasm, or trauma) were retrospectively investigated for clinical presentation, imaging features, treatment outcomes, and complications. Technical success included delivery of embolic agent with complete obliteration of fistula. Clinical success included resolution of symptoms and freedom from recurrence and/or reintervention. Renal parenchymal loss was estimated by postembolization angiography and categorized as 0%, <25%, 25%-50%, or >50%.
  
  
  RESULTS
  Twenty-five patients were referred with a presumptive diagnosis of intraparenchymal renal artery aneurysms. Of these, 10 had true intrarenal aneurysms, three had angiomyolipomas, and 12 had CRAVFs (mean age, 54; range, 29-71 years; eight women). Presenting symptoms included hematuria (eight gross, eight microscopic), refractory hypertension (diastolic blood pressure ≥ 90 mm Hg despite three or more medications; n = 6), flank pain (n = 8), high-output state (HOS; featuring tachycardia and jugular venous distention; n = 3), and flank bruit (n = 1). Defining angiographic features included a high-flow AVF fed by a single, enlarged intrarenal branch shunting into an aneurismal draining vein, occasionally featuring a calcified rim (four patients). All patients underwent transarterial embolization with coils (n = 5), coils and n-butylcyanoacrylate (n = 3), detachable balloons (n = 2), or Amplatzer plugs (n = 2). Technical success was 100%. Hematuria, tachycardia, jugular venous distension, pain, and bruit resolved in all. Hypertension improved in four of six patients (required less than three medications postembolization). Complications included postembolization syndrome in nine patients. Parenchymal loss was limited to <25% and observed in five patients without development of acute kidney injury or worsening hypertension. There were no recurrences or reinterventions at a mean follow-up of 55 months (range, 5-96 months). There was one death at 8 years follow-up from intercurrent coronary disease in a patient without high-output state.
  
  
  CONCLUSIONS
  With greater awareness and accurate diagnosis, effective and durable transarterial treatment of CRAVFs can be safely performed.
authors.affiliation: Lenox Hill Heart and Vascular Institute of New York, North Shore-LIJ Healthcare System, New York, NY. Electronic address: naiemn@gmail.com.
authors.forename: Naiem
authors.initials: N
authors.lastname: Nassiri
authors.forename: Yuriy
authors.initials: Y
authors.lastname: Dudiy
authors.forename: Alfio
authors.initials: A
authors.lastname: Carroccio
authors.forename: Robert J
authors.initials: RJ
authors.lastname: Rosen
mesh_terms: D000328:Adult; D000368:Aged; D015901:Angiography, Digital Subtraction; D001164:Arteriovenous Fistula / Q000175:diagnosis / Q000401:mortality / Q000503:physiopathology / Q000628:therapy*; D004621:Embolization, Therapeutic / Q000009:adverse effects / Q000295:instrumentation / Q000401:mortality; D004659:Enbucrilate / Q000008:administration & dosage; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D010690:Phlebography; D011237:Predictive Value of Tests; D012039:Regional Blood Flow; D012077:Renal Artery / Q000002:abnormalities* / Q000000981:diagnostic imaging / Q000503:physiopathology; D012079:Renal Circulation; D012082:Renal Veins / Q000002:abnormalities* / Q000000981:diagnostic imaging / Q000503:physiopathology; D012189:Retrospective Studies; D013997:Time Factors; D016896:Treatment Outcome; D055815:Young Adult
chemical_list: D004659:Enbucrilate